Anti-Complement C4A Antibody (CAB3545)

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SKU:
CAB3545
Product type:
Antibody
Antibody Type:
Monoclonal Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
C4
Synonyms:
C4A2
Synonyms:
C4A3
Synonyms:
C4A4
Synonyms:
C4A6
Synonyms:
C4AD
Synonyms:
C4S
Synonyms:
CO4
Synonyms:
CPAMD2
Synonyms:
RG
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Description

Product Name:Complement C4A Rabbit mAb
Product Code:CAB3545
Size:20uL, 50uL, 100uL
Synonyms:C4, C4A2, C4A3, C4A4, C4A6, C4AD, C4S, CO4, CPAMD2, RG
Applications:WB, IF
Reactivity:Human
Host Species:Rabbit
Immunogen:A synthesized peptide derived from human Complement C4A
Applications:WB, IF
Recommended Dilutions:WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:Human
Positive Samples:HepG2
Immunogen:A synthesized peptide derived from human Complement C4A
Purification Method:Affinity purification
Storage:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:720
Uniprot:P0C0L4
Calculated MW:193kDa
Observed MW:200KDa
UniProt Protein Function:C4A: C4 plays a central role in the activation of the classical pathway of the complement system. It is processed by activated C1 which removes from the alpha chain the C4a anaphylatoxin. The remaining alpha chain fragment C4b is the major activation product and is an essential subunit of the C3 convertase (C4b2a) and the C5 convertase (C3bC4b2a) enzymes of the classical complement pathway. Defects in C4A are the cause of complement component 4A deficiency (C4AD). A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis. Defects in C4A are a cause of susceptibility to systemic lupus erythematosus (SLE). A chronic, inflammatory and often febrile multisystemic disorder of connective tissue. It affects principally the skin, joints, kidneys and serosal membranes. It is thought to represent a failure of the regulatory mechanisms of the autoimmune system. Interindividual copy- number variation (CNV) of complement component C4 and associated polymorphisms result in different susceptibilities to SLE. The risk of SLE susceptibility has been shown to be significantly increased among subjects with only two copies of total C4. A high copy number is a protective factor against SLE.
UniProt Protein Details:

Protein type:Inhibitor; Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 6p21.3

Cellular Component: extracellular region; extracellular space; plasma membrane

Molecular Function:complement component C1q binding; endopeptidase inhibitor activity

Biological Process: complement activation; complement activation, classical pathway; inflammatory response; innate immune response; regulation of complement activation

Disease: Blood Group, Chido/rodgers System; Complement Component 4a Deficiency

NCBI Summary:This gene encodes the basic form of complement factor 4, part of the classical activation pathway. The protein is expressed as a single chain precursor which is proteolytically cleaved into a trimer of alpha, beta, and gamma chains prior to secretion. The trimer provides a surface for interaction between the antigen-antibody complex and other complement components. The alpha chain may be cleaved to release C4 anaphylatoxin, a mediator of local inflammation. Deficiency of this protein is associated with systemic lupus erythematosus. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. Varying haplotypes of this gene cluster exist, such that individuals may have 1, 2, or 3 copies of this gene. In addition, this gene exists as a long form and a short form due to the presence or absence of a 6.4 kb endogenous HERV-K retrovirus in intron 9. [provided by RefSeq, Jul 2008]
UniProt Code:P0C0L4
NCBI GenInfo Identifier:476007827
NCBI Gene ID:721
NCBI Accession:P0C0L4.2
UniProt Secondary Accession:P0C0L4,P01028, P78445, Q13160, Q13906, A6H8M8, A6NHJ5 A7E2V2, B0QZR6, B0V2C8, B2RUT6, B7ZVZ6,
UniProt Related Accession:P0C0L5
Molecular Weight:187,704 Da
NCBI Full Name:Complement C4-A
NCBI Synonym Full Names:complement component 4B (Chido blood group)
NCBI Official Symbol:C4B  
NCBI Official Synonym Symbols:CH; C4F; CO4; C4B1; C4B2; C4B3; C4B5; C4BD; C4B12; C4B_2; CPAMD3  
NCBI Protein Information:complement C4-B
UniProt Protein Name:Complement C4-A
UniProt Synonym Protein Names:Acidic complement C4; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 2
Protein Family:Complement C4
UniProt Gene Name:C4A  
UniProt Entry Name:CO4A_HUMAN
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